伯纳德-索利尔综合征中缺乏药物依赖性抗体的血小板受体。
Absence of the platelet receptor for drug-dependent antibodies in the Bernard-Soulier syndrome.
作者信息
Kunicki T J, Johnson M M, Aster R H
出版信息
J Clin Invest. 1978 Sep;62(3):716-9. doi: 10.1172/JCI109181.
Abstract
The platelet membrane receptor for quinidine- and quinine-dependent antibodies was studied in three patients with the Bernard-Soulier syndrome (BSS) and in normal subjects with immunologic techniques based on the release of 51Cr from labeled platelets. The receptor could not be detected on BSS platelets but was present on platelets from each of 180 normal subjects. BSS platelets reacted normally with other allo- and autoantibodies. In confirmation of previous reports, BSS platelets were found to be deficient in glycoproteins Ib and Is. However, after apparently total cleavage of these proteins from the membrane of normal platelets by controlled hydrolysis with trypsin or chymotrypsin, 80% of the drug-dependent antibody receptor activity was retained. These observations suggest the existence of an additional, hitherto unrecognized membrane defect in Bernard-Soulier platelets.
摘要
采用基于从标记血小板中释放⁵¹Cr的免疫技术，对3例伯-苏综合征（BSS）患者及正常受试者的奎尼丁和奎宁依赖性抗体的血小板膜受体进行了研究。在BSS血小板上未检测到该受体，但在180名正常受试者的血小板上均有存在。BSS血小板与其他同种异体和自身抗体反应正常。正如先前报道所证实的，发现BSS血小板中糖蛋白Ib和Is缺乏。然而，在用胰蛋白酶或糜蛋白酶通过可控水解从正常血小板膜上明显完全裂解这些蛋白质后，仍保留了80%的药物依赖性抗体受体活性。这些观察结果提示，伯-苏血小板中存在一种迄今未被认识的额外膜缺陷。
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